Bone marrow transplantation for constitutional pure red cell aplasia

Autor:	J Lazerson, Y Barak, Eva C. Guinan, Kenneth I. Weinberg, K Williams, PP Dukes, C Lenarsky, Howard J. Weinstein, Stefan Siegel, Jorge A. Ortega
Rok vydání:	1988
Předmět:	medicine.medical_specialty Cyclophosphamide Red Cell business.industry Lymphocyte Immunology Pure red cell aplasia Cell Biology Hematology Procarbazine medicine.disease Biochemistry Surgery Haematopoiesis medicine.anatomical_structure medicine Erythropoiesis business Busulfan medicine.drug
Zdroj:	Blood. 71:226-229
ISSN:	1528-0020 0006-4971
Popis:	Constitutional pure red cell aplasia (CPRCA) is a syndrome of failed erythropoiesis usually diagnosed within the first year of life. Four patients with CPRCA received transplants with marrow from their HLA- identical, mixed lymphocyte culture-nonreactive siblings. All patients were resistant to corticosteroid therapy and were dependent on regular red cell transfusions for at least 5 years. Three patients were conditioned with procarbazine, antithymocyte globulin, cyclophosphamide, and busulfan, and one was conditioned with antithymocyte serum, cyclophosphamide, and busulfan. Three patients promptly had successful engraftments with establishment of donor hematopoiesis. One patient initially rejected his graft but received a successful retransplant. All patients are currently alive with Karnofsky performance scores of 100 and normal erythropoiesis of donor origin. Despite a history of multiple transfusions, bone marrow transplantation is a potentially curative therapy for patients with CPRCA.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e9d49e28d6f1a0c535e62336f59adab0 https://doi.org/10.1182/blood.v71.1.226.bloodjournal711226 Zobrazit plný text záznamu